[P1-39] Spinal Posterior Reversible Encephalopathy Syndrome mimicking Long cord myelitis
[Case History]: An 11 years old girl presented with acute ascending symmetric weakness over 5 days along with bowel and bladder incontinence. She had history of recurrent urinary tract infection. At admission, she was conscious with blood pressure of 152/120 mm Hg and fundus showing retinal hemorrhages. Echocardiography showed normal ejection fraction of 55-60%. She was started on sodium nitroprusside for hypertensive emergency.
For suspected transverse myelitis, she was also treated with pulse methyl prednisolone which was given after control of blood pressure. Her weakness progressed over next 2 days necessitating ventilation. IVIG was given at 2gm/kg. Anti aquaporin-4 antibodies and workup for systemic lupus erythematosis was negative. MRI spine showed long segment central hyperintensity patchy involvement, extending from cervical level with evidence of hemorrhage in lumbosacral region. MRI brain showed bilateral periventricular white matter hyperintensities. Basal ganglia showed bilateral putaminal cystic change with hemorrhagic residue and hemorrhagic infarcts in the brainstem. CT angiography of abdomen, neck and brain vessels was normal. Skin biopsy was not suggestive of vasculitis. Urine for metanephrines was negative and plasma renin activity was normal
[Discussion]: Posterior Reversible Encephalopathy Syndrome (PRES) syndrome is a clinical syndrome with hypertensive emergency and encephalopathy due to brain involvement due to posterior cerebral white matter, basal ganglia and brainstem involvement. Similarly, changes in spinal cord due to severe hypertension described as PRES Spinal Cord have been described which may mimic long segment myelitis. Clinical recognition of this entity may change entire management and prevent excessive immunomodulation.
For suspected transverse myelitis, she was also treated with pulse methyl prednisolone which was given after control of blood pressure. Her weakness progressed over next 2 days necessitating ventilation. IVIG was given at 2gm/kg. Anti aquaporin-4 antibodies and workup for systemic lupus erythematosis was negative. MRI spine showed long segment central hyperintensity patchy involvement, extending from cervical level with evidence of hemorrhage in lumbosacral region. MRI brain showed bilateral periventricular white matter hyperintensities. Basal ganglia showed bilateral putaminal cystic change with hemorrhagic residue and hemorrhagic infarcts in the brainstem. CT angiography of abdomen, neck and brain vessels was normal. Skin biopsy was not suggestive of vasculitis. Urine for metanephrines was negative and plasma renin activity was normal
[Discussion]: Posterior Reversible Encephalopathy Syndrome (PRES) syndrome is a clinical syndrome with hypertensive emergency and encephalopathy due to brain involvement due to posterior cerebral white matter, basal ganglia and brainstem involvement. Similarly, changes in spinal cord due to severe hypertension described as PRES Spinal Cord have been described which may mimic long segment myelitis. Clinical recognition of this entity may change entire management and prevent excessive immunomodulation.